Polycystic kidney disease (PKD) is a genetic disorder in which cysts develop in your kidneys. These cysts cause your kidneys to enlarge and may lead to damage.
There are two main types of PKD: autosomal dominant polycystic kidney disease (ADPKD) and autosomal recessive polycystic kidney disease (ARPKD).
Both ADPKD and ARPKD are caused by abnormal genes, which may be passed from parent to child. In rare cases, the genetic mutation occurs spontaneously in someone with no family history of the disease.
Take a moment to learn about the differences between ADPKD and ARPKD.
ADPKD and ARPKD differ from one another in several key ways:
- Incidence. ADPKD is far more common than ARPKD. Roughly 9 in 10 people with PKD have ADPKD, reports the American Kidney Fund.
- Pattern of inheritance. To develop ADPKD, you only need to inherit one copy of the mutated gene that’s responsible for the disease. To develop ARPKD, you must have two copies of the mutated gene — with one copy inherited from each parent, in most cases.
- Age of onset. ADPKD is often known as “adult PKD” because signs and symptoms tend to develop between the ages of 30 and 40. ARPKD is often known as “infantile PKD” because signs and symptoms appear early in life, shortly after birth or later in childhood.
- Location of cysts. ADPKD often causes cysts to develop only in the kidneys, while ARPKD often causes cysts to develop in the liver and the kidneys. People with either type may also develop cysts in their pancreas, spleen, large bowel, or ovaries.
- Severity of disease. ARPKD tends to cause more severe symptoms and complications to develop earlier in life.
Over time, ADPKD or ARPKD can damage your kidneys. This may cause chronic pain in your side or back. It may also stop your kidneys from working properly.
If your kidneys stop working well, it can lead to a toxic buildup of waste in your blood. It can also cause kidney failure, which requires lifelong dialysis or a kidney transplant to treat.
ADPKD and ARPKD may also cause other potential complications, including:
- high blood pressure, which may further damage your kidneys and raise your risk of stroke and heart disease
- preeclampsia, which is a potentially life threatening form of high blood pressure that may develop in pregnancy
- urinary tract infections, which develop when bacteria enter your urinary tract system and grow to harmful levels
- kidney stones, which form when minerals in your urine crystalize into hard deposits
- diverticulosis, which occurs when weak spots and pouches develop in the wall of your large intestine
- mitral valve prolapse, which occurs when a valve in your heart stops closing properly and allows blood to leak backward
- brain aneurysm, which occurs when a blood vessel in your brain bulges and puts you at risk of a brain bleed
ARPKD tends to cause more serious symptoms and complications earlier in life, compared with ADPKD. Children who are born with ARPKD may have high blood pressure, trouble breathing, difficulty keeping food down, and impaired growth.
Babies with severe cases of ARPKD may not live for more than a few hours or days after birth.
To help slow the development of ADPKD, your doctor may prescribe a new type of medication known as tolvaptan (Jynarque). This medication has been shown to slow the progression of the disease and reduce the risk of kidney failure. It’s not approved to treat ARPKD.
To help manage potential symptoms and complications of either ADPKD or ARPKD, your doctor may prescribe any of the following:
- dialysis or kidney transplant, if you develop kidney failure
- blood pressure medication, if you have high blood pressure
- antibiotic medication, if you have a urinary tract infection
- pain medication, if you have pain caused by cysts
- surgery to remove cysts, if they’re causing severe pressure and pain
In some cases, you may need other treatments to help manage complications of the disease.
Your doctor will also encourage you to practice healthy lifestyle habits to help keep your blood pressure in check and reduce your risk of complications. For example, it’s important to:
- eat a nutrient-rich diet that’s low in sodium, saturated fats, and added sugars
- get at least 30 minutes of moderate-intensity exercise on most days each week
- keep your weight within a healthy range
- limit your alcohol consumption
- avoid smoking
- minimize stress
PKD can shorten a person’s life span, especially if the disease isn’t managed effectively.
Roughly 60 percent of people with PKD develop kidney failure by age 70, reports the National Kidney Foundation. Without effective treatment with dialysis or kidney transplant, kidney failure tends to cause death within a few days or weeks.
ARPKD tends to cause serious complications at a younger age than ADPKD, which substantially reduce life expectancy.
According to the American Kidney Fund, about 30 percent of babies with ARPKD die within a month of birth. Among children with ARPKD who survive beyond the first month of life, about 82 percent live to age 10 or older.
Talk to your doctor to get a better understanding of your outlook with ADPKD or ARPKD.
There’s no cure for ADPKD or ARPKD. However, treatments and lifestyle habits may be used to manage symptoms and reduce the risk of complications. Research is ongoing into therapies to address the condition.
Although ADPKD and ARPKD both cause cysts to develop in the kidneys, ARPKD tends to cause more severe symptoms and complications earlier in life.
If you have ADPKD or ARPKD, your doctor may prescribe medications, lifestyle changes, and other treatments to help manage symptoms and potential complications. The conditions have some important differences in symptoms and treatment options, so it’s important to understand which condition you have.
Talk to your doctor to learn more about your treatment options and outlook.